The distribution of germline SDHX gene variants associated with dSDH-wtGIST differs from that seen in the tumour types most commonly associated with dSDH (phaeochromocytoma and paranglioma (PPGL) and head and neck paraganglioma) in that germline SDHA variants are frequent with wtGIST but an infrequent cause of PPGL or head and neck paraganglioma [7, 9]. The gene discussed is SDHA; the disease is head and neck paraganglioma.